Management of Interrupted Aortic Arch

Area of Science:

• Pediatric Cardiology
• Congenital Heart Surgery
• Neonatal Intensive Care

Background:

• Interrupted aortic arch (IAA) is a critical congenital heart defect.
• Prostaglandin E1 (PGE1) significantly improved IAA management since the late 1970s.
• Current management emphasizes staged resuscitation and primary neonatal surgical repair.

Purpose of the Study:

• To review the current management strategies for interrupted aortic arch.
• To highlight the long-term outcomes and potential complications following surgical repair.
• To emphasize the importance of developmental surveillance in affected neonates.

Main Methods:

• Review of historical and current management protocols for IAA.
• Analysis of surgical techniques including one-stage repair, ventricular septal defect closure, and selective cerebral perfusion.
• Discussion of late complications such as left ventricular outflow tract obstruction and associated interventions.

Main Results:

• One-stage primary neonatal repair with direct arch anastomosis and VSD closure is the standard surgical approach.
• Selective cerebral perfusion with near-infrared monitoring is increasingly utilized.
• Late left ventricular outflow tract obstruction is a significant concern, often requiring reintervention, but initial neonatal management should avoid subaortic stenosis procedures.

Conclusions:

• While physiologically corrective, IAA repair necessitates long-term follow-up due to high rates of late left ventricular outflow tract obstruction.
• Developmental follow-up is crucial due to the frequent association with DiGeorge syndrome.
• Management requires a multidisciplinary approach, balancing immediate surgical needs with long-term cardiac and neurodevelopmental outcomes.